CASE REPORT Uterus didelphys, hemihydrocolpos and empty ipsilateral lumbar fossa in a newborn girl: involuted dysplastic kidney rather than renal agenesis Pierre-Hugues Vivier & Agnès Liard & Marion Beurdeley & Marie Brasseur-Daudruy & Cécile Cellier & Patrick Le Dosseur & Jean-Nicolas Dacher Received: 22 November 2010 / Accepted: 12 December 2010 / Published online: 25 June 2011 # Springer-Verlag 2011 Abstract We report a case of Herlyn-Werner-Wunderlich syndrome diagnosed in the neonatal period. US revealed the classic association of a uterus didelphys with blind hemivagina and no ipsilateral kidney. The diagnosis was established by postnatal US and confirmed by MRI. Differential diagnoses are discussed. A trans-hymeneal resection of the vaginal septum was performed at 1 month of age. Intra operative endoscopy revealed no left hemitrigone but showed an atretic orifice in the ipsilateral blind hemivagina, probably corresponding to the insertion of an ectopic ureter. Follow-up was unremarkable. Keywords Genitalia . Female abnormality . Urogenital abnormality . Multicystic dysplastic kidney . Hydrocolpos Introduction Uterus didelphys with blind hemivagina and ipsilateral absent kidney is a rare congenital malformation. This association is also known as Herlyn-Werner-Wunderlich syndrome. It is usually diagnosed after menarche [1]. We report a case diagnosed in the neonatal period, discuss potential differen- tial diagnoses, and describe its management. Case report A 5-day-old girl was referred to our institution for abdominal US to confirm the prenatal diagnosis of a dilated left pelvic kidney (Fig. 1). She was a term baby, and the delivery had been unremarkable. The right kidney was enlarged with a length of 65 mm but no other sonographic abnormality was present. US confirmed the absence of a kidney in the left lumbar fossa but also in the pelvis. However, US revealed a retrovesical fluid-filled structure 4 cm in length, with internal echoes/debris delineated by a visible wall (Fig. 2). A uterus didelphys was also present (Fig. 3) with a communication between the left hemiuterus and the fluid-filled structure (Fig. 4). Based on the association of these anomalies, the diagnosis of Herlyn- Werner-Wunderlich syndrome was made. As pre- and post- natal examinations were discrepant, the paediatric surgeon requested a complementary MRI examination (Fig. 5), which did not display a left kidney or any renal remnant, and confirmed the sonographic diagnosis. The patient underwent a trans-hymeneal surgical resection of the vaginal septum at the age of 1 month. An endoscopy performed during the intervention showed a right ureteric orifice in the bladder without left hemitrigone. A light liquid discharged from the bulging blind hemivagina after incision. Vagino- scopy performed after resection of the anterior part of the septum confirmed the presence of one hemicervix on each side of the posterior part of the septum. On the lateral aspect of the left (anteriorly obstructed) hemivagina, an atretic orifice was visible (Fig. 6), supposedly a remnant of the ectopic insertion of the left ureter. This atretic orifice could not be catheterised or opacified (Fig. 7). However, this intra operative endoscopy allowed better delineation of the remaining septum prior to its resection. The child was P.-H. Vivier (*) : M. Brasseur-Daudruy : C. Cellier : P. Le Dosseur : J.-N. Dacher Pediatric and Foetal Imaging Service, Department of Radiology, CHU Charles Nicolle, Rouen University Hospital, 1 rue de Germont, 76031 Rouen Cedex, France e-mail: pierre-hugues.vivier@chu-rouen.fr A. Liard : M. Beurdeley Department of Pediatric Surgery, University Hospital, 1 rue de Germont, 76031 Rouen Cedex, France Pediatr Radiol (2011) 41:1205–1207 DOI 10.1007/s00247-011-2046-y